Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
17 Cards in this Set
- Front
- Back
|
def
|
clonal stem cell defect
may cause aplastic anemia myelodysplasia or acute leukemia increased sens to acidosis |
|
|
what def
|
def of complement regulator protein CD55 CD59-- decay accelerating factor or homologius restriction factor ( CD59)
gene for phosphotidil inositol class A PIG-A os defective>> to over activation of complement system during sleep-- relative hypoventilation lead to mild increased in Pco2 and acidosis noting to patint but PNG lead to hemolysis and thrombosis |
|
|
what is most likely diagnosis
|
episodic dark urine in the firs at urination
pancytopenia and iron def anemia |
|
|
what is MCC of death in PNH
|
thrombosis
|
|
|
symptoms
|
dark urine
jaundice pallor veno occlusive events abdominal back pain |
|
|
ds
|
chronic hemolysis
hemoglobinuria after period os sleep pancytopenia increasd LDH decreas to abstent hemoglobin hemosiderinuria hams test sucrose hemolysis test decreased to absent iron stores |
|
|
what is most spec test
|
ham test
|
|
|
what hams and surose test to do
|
they activate comp pathway in vitro
|
|
|
if yo uhave answe CD55 Cd59
|
no ham test and sucrose in y our answer!!
|
|
|
what is another way to say about cd55 cd59
what is the gold standart |
flow cytometry numb 1 diagnostic test
|
|
|
tx
|
steroid
folate iron tranfusion during episode anticoag for thrombsis androgens |
|
|
best initial therapy
|
prednison
|
|
|
metod to cure
|
only allogenic BM transplt
|
|
|
what drug will be working n cd55
|
eculizumab inactivate c 5 in the compliment pathway and decreased cell destruction
this drug is compliment inactivate mechanism of PNH is compl overactivation |
|
|
where will be thrombosis
|
large vessels
mesent and hepatic veins- most common |
|
|
risk of tromosis is ^^^^ and what will be in the liver
|
budd choary syndrome from circul thromogenic particles
|
|
|
intravascular hemolysis
|
intravascular hemolysis->free Hb released and immediately bond to haptoglobin( synthesized in liver), forming the complex. that removed by macrophages in the speeln liver and BM ( low haptoglobin !!)
liver cant match the loss of haptoglobin and free HB is filtered into urine(hemoglobinuria!!!!) some of free Hb is reabsorb by kideny-> and eventually is lost in urine- hemosiderinuria |
